Enamel defects observed in primary anterior teeth of 124 children with congenital cerebral palsy (CP) born 1983 through 1985 in four northern California counties were categorized using an adaptation of the Developmental Defects of Enamel Index. Photographs of anterior teeth and abstracts of medical records with no identifiers were used for the purpose. Nineteen children (15%) had crowns or loss of tooth substance due to attrition (LTS). Missing enamel including horizontal groove (ME), was observed in 40 children (32%). Twenty-four children without ME (19%) had enamel pits, vertical grooves, or colored enamel opacities. Forty-one (33%) had clinically normal enamel. ME children did not differ significantly from those with normal enamel with respect to race, sex, singleton vs twin, severity of CP, or presence of dysmorphic features. ME children were more often low in gestational age, and even those children who were not low in birth weight, were more often reported by parents to have required neonatal intensive care.